More people are dying from valley fever than previously thought, and illnesses including diabetes, lung disease, arthritis and certain cancers may increase a person's chances of dying from the disease, according to a new study.
This past year, researchers have puzzled over the rise of valley fever cases. Diagnosed cases have grown from 1,200 in 1995 to more than 20,000 in 2011, according to the Centers for Disease Control. Now researchers have new evidence to consider: a study to be published in the November issue of the CDC's Emerging Infectious Diseases journal.
There were 3,089 documented deaths nationwide that valley fever -- also known as coccidioidomycosis -- was an underlying or contributing cause of between 1990 and 2008, an average of about 170 deaths every year, according to the researchers' study of data pulled from death certificates. That's nearly twice the number reported by the CDC in the past, which has recorded as few as 73 in 2003 to as high as 100 in 2004.
"Coccidioidomycosis remains a major cause of death in the United States," wrote Jennifer Y. Huang, who authored the study as her master's thesis at the Keck School of Medicine at the University of Southern California along with researchers from UCLA's Fielding School of Public Health.
"Given the growing U.S. population of elderly and immunosuppressed persons, the number of coccidioidomycosis-related deaths will probably increase, resulting in higher costs to the healthcare system."
Dr. Benjamin Bristow, a public health physician and one of the study's authors, said the paper shows there are "definitely certain groups that are more likely to die from valley fever" and the numbers shed light on those disparities.
"We're finally beginning to get a sense of how real the problem is," Bristow said.
Valley fever experts said the hard numbers on valley fever-related deaths could finally start to draw attention to the disease.
Valley fever is often referred to as an "orphan disease" that isn't very prevalent. But Dr. John Galgiani, director of the Valley Fever Center for Excellence at the University of Arizona, said the study could put a dent into that way of thinking.
"This disease was overwhelming, and it's anything but an orphan disease" for those who died, Galgiani said.
The study cements the prevalence of valley fever and draws attention to the fact that, despite its health impact, the disease has not drawn significant federal or private research funding, said Dr. George Rutherford, professor of epidemiology and preventive medicine at the University of California, San Francisco.
"There are few infectious diseases for which we tolerate this kind of mortality," he said.
Rutherford said the data establishes a base line to compare trends in the future.
"Just from a data standpoint, having this line in the sand is a good thing," he said.
The research is limited by the fact that valley fever is often not diagnosed or misdiagnosed as another disease. Researchers noted in the study that the death certificates their data were drawn from "probably underreport causes of death and can contain errors."
"I think what we're demonstrating is really the lower end of the reality," said Bristow. "My guess is there are a lot more valley fever deaths and cases that we're not finding in the data."
VALLEY FEVER STRIKES ELDERLY, WEAKENED
The aging of the U.S. population and the rise in chronic illnesses could drive an increase in valley fever-related deaths, the researchers found. The fungus that causes valley fever is mainly found in California and Arizona, and the researchers attribute the high concentration of deaths in those states to the fact that the valley fever fungus is common there and the states are "classic retirement magnets."
California and Arizona "attract elderly persons to migrate and settle down, thereby introducing new, unexposed populations" to the fungus, they wrote.
People spending their vacations or entire winters in those states before going back to their hometowns may also be bringing the disease with them. Researchers noted that people died from valley fever in every state, "which probably reflects population mobility and movement in and out of coccidioidomycosis-endemic areas after exposure."
The study found that more than one-third of the deaths associated with valley fever were people between ages 65 and 84.
Researchers also found that there were certain health conditions more often associated with the disease. The most common was diabetes, with 384 deaths that listed both diabetes and coccidioidomycosis on the death certificate. That was followed by chronic obstructive pulmonary disease, HIV infection and non-Hodgkin lymphoma.
"The conditions that were associated with coccidioidomycosis were all inherently associated with immunosuppression: HIV, tuberculosis, diabetes mellitus, autoimmune diseases, organ transplant and cancers of lymphatic cells," Huang and colleagues wrote.
The connection between valley fever and people whose immune systems are already weakened by aging or other diseases is no surprise, said Dr. Navin Amin, an infectious disease specialist and chairman of family medicine and pediatrics at Kern Medical Center. "We used to have a lot of HIV people who would get cocci and if it wasn't picked up early, they would die," Amin said. "Every HIV patient we had, when they developed cocci, it spread like wildfire."
Amin said he suspects the correlation between valley fever and lupus and rheumatoid arthritis found in the study could be caused by the medications patients take for those two conditions that weakened their immune system.
Researchers also looked at valley fever-related deaths across different race and ethnic groups. While the majority of deaths were among white people, the study found that people of other ethnicities, particularly Native Americans and Hispanics, had the highest mortality rate. Native Americans were about six times more likely to have valley fever appear on their death record than white people, and Hispanics were about four times more likely to have the disease listed on their death record, the study said.
Researchers wrote that the high rates of valley fever-related deaths among Native Americans could be tied to cultural practices and exposure to dust. Health care could play a part as well, they wrote.
"Poor access to health care services might delay diagnosis, resulting in more severe disease," they wrote.
But researchers also cautioned that Native Americans' high rates should be viewed carefully because they accounted for a small number -- just 79 -- of the total deaths.
Still, Bristow said it's important to find the disparities in the groups the disease affects so those discrepancies can be addressed.
"I think we have a duty to really put our efforts toward those that are most impacted by illness," Bristow said, adding that those people often have less access to other resources such as health care and housing.
DEATH TOLL LIKELY TO RISE
The study's researchers concluded that valley fever-related deaths will probably rise.
"One can probably expect to see more valley fever in the future because we're seeing more people with problematic immune systems," Bristow said.
An increase in instances of valley fever means more health care costs, researchers wrote, pointing to the costs of the disease in epidemic states as an example. The researchers noted that "almost half of the reported case-patients are hospitalized and make multiple visits to emergency rooms and outpatient facilities during the course of the illness."
"There are very significant cost implications of this infection. It tends to be very costly to treat," said Shira Shafir, assistant professor of epidemiology at the Fielding School of Public Health at UCLA and one of the study's authors. "When we have so much of this, those costs really add up."
Researchers wrote that doctors should recognize the heightened risk of valley fever-related deaths among the elderly, men, Hispanics, Native Americans and people with compromised immune systems.
Rutherford said he hoped the study would bring renewed national attention to the disease and lead to targeted funding for a vaccine and the development of new treatments.
D. Rebecca Prevots, chief of the epidemiology unit at the National Institute of Allergy and Infectious Diseases, said the report fills a gap in research on valley fever and mortality. The study stands out, she said, because it is national in scope, covers an 18-year span and highlights the racial and ethnic disparities within the disease.
"I think it is an important work that calls attention to the issue of cocci and deaths from cocci," Prevots said.
Bristow said that it is now up to physicians, public health officials and policymakers to look at the burden from the disease and design an appropriate response.
"Understanding the problem is the first step, and that's what this paper helps us do," Bristow said. "This gives valley fever a voice. This says, 'Valley fever is real, it is present, it is killing Americans and it deserves attention.'"
The Reporting on Health Collaborative involves The Californian, Merced Sun-Star, Radio BilingÃ¼e in Fresno, The Record in Stockton, Valley Public Radio in Fresno and Bakersfield, Vida en el Valle in Fresno, the Voice of OC in Santa Ana and ReportingonHealth.org.
It's an initiative of The California Endowment Health Journalism Fellowships at USC's Annenberg School for Communication and Journalism.